I am a plastic surgeon in Little Rock, AR. I used to "suture for a living", I continue "to live to sew". These days most of my sewing is piecing quilts. I love the patterns and interplay of the fabric color. I would like to explore writing about medical/surgical topics as well as sewing/quilting topics. I will do my best to make sure both are represented accurately as I share with both colleagues and the general public.

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Monday, September 29, 2008

Complex Regional Pain Syndrome (CRPS) is a multi-symptom, multi-system syndrome that remain poorly understood. It was called reflex sympathetic dystrophy (RSD) when I first learned about it. I admit I still tend to call it RSD.

Historical Review

In 1864 Silas Weir Mitchell published his findings on gunshot wounds of nerves in a now classic article (2nd ref below). Then in 1867, Mitchell called this condition causalgia from the Greek word meaning "burning pain". Mitchell described the condition well, but was unaware of the etiologic connection to the sympathetic nervous system. Over the years other authors who did make the connection of the vascular and nervous systems in this condition have suggested names such as neurovascular dystrophy, post-traumatic vasomotor disorders, sympathetic neurovascular dystrophy, post-traumatic vasospasm, postinfarctional sclero-dactyly, traumatic angiospasm, causalgic state, minor causalgia, mimo-causalgia, and Sudeck's atrophy. It was in 1967 after Richards article (3rd reference) that the term reflex sympathetic dystrophy began to be used.

In 1995, the International Association for the Study of Pain (IASP) decided that the terms complex regional pain syndrome (CRPS) type I and type II were better than the respective names reflex sympathetic dystrophy and causalgia. The term "Complex" was added to convey the reality that this condition expresses varied signs and symptoms.

What is RSD / CRPS?

It is a condition that does not follow the normal healing path after an injury to a nerve or soft tissue. Development of RSD / CRPS does not correlate to the magnitude of the initial injury. The reasons that the sympathetic nervous systems assumes an abnormal function after an injury is still not understood. (photo credit)

The International Association for the Study of Pain (IASP) lists the diagnostic criteria for complex regional pain syndrome I (CRPS I) (RSDS) as follows:

The presence of an initiating noxious event or a cause of immobilization

Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia disproportionate to the inciting event

Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain

The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.

According to the IASP, CRPS II (also known as causalgia) is diagnosed as follows:

The presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve

Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain

The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.

The primary difference between type I and type II is the identification of a definable nerve injury. For more information check this eMedicine article and this website (RSD Foundation). You will also find a nice video animation on the RSD Foundation site that shows how an injury might trigger RSD / CRPS.

TREATMENT

The cornerstone in the treatment of RSD / CRPS is normal use of the affected part as much as possible. This is done through education, pain control, and physical therapy. (photo credit)

9 comments:

I am afflicted with RSD/CRPS. The way I explain it is that my nerves turned on after an accident and never turned back off. It's quick and people tend to understand that more than trying to explain in more detail. Then I tell them it's called RSD/CRPS, in complete terms of course.

GREAT post, thank you!I'll be linking to it from my blog, as I've found that a lot of people are curious about it. One of the newer treatments that can be helpful for CRPS is mirrorbox and/or motor imagery - the main proponent of this is Lorimer Moseley - our experience is that this approach can be helpful for some people in the earlier stages, paired with graded reactivation. Johan Vlaeyen and colleagues have also used exposure therapy similar to that used for phobia. CheersBronniehttp://healthskills.wordpress.com

I am grateful for every intelligent post on the internet about CRPS. (I play the "acronym" game, although, as you noted, most people are more comfortable with the more inaccurate "RSD.") I have both Type 1 and Type 2 -- primarily 2 -- but have found that there are few who understand the distinction. Initially in my right leg and left arm, due to nerve and orthopedic injuries, when it "spread," most of the newly afflicted areas were Type 1. Now, any orthopedic insult usually results in a spread -- and since I have avascular necrosis in almost all joints, my odds for injury and spread are high. Yadda yadda! To all the doctors out there: *please* keep an open mind whenever you see severe sensitivity, pain "out of proportion," color and temperature variation, etcetera. Early diagnosis is key, is crucial. My life is quite ruined -- I wasn't diagnosed until 21 months out from my injuries (the doctors had tunnel vision due to all the orthopedic schtuff...), and now, CRPS has a firm hold in all four extremities, and even in a small area around my chin (that *freaks* me out!). Pain management is crucial, and one of the greatest gifts I was given by the medicos was recognition that the intense physical therapy (really the only hope, at this juncture) would be impossible unless my pain levels were addressed. Anyway, a huge THANK YOU for posting about CRPS. Cough... okay, and for posting about *RSD*! If you or others wish to contact those docs on the cutting edge, I would recommend Dr. Anthony Kirkpatrick, founder of the RSD / CRPS Treatment Center and Research Institute in Tampa, and Dr. Robert Schwartzman, chair of neurology at Drexel University, in Philadelphia. Both are dedicated to finding real treatment for this sucky disease and are involved in the current investigation into the ketamine "coma" protocol being researched in Germany and Mexico. (Oh, if I had the money!) I will also link your blog to my tangential list of blog favorites, unless you object. Feel free to object -- I won't be offended!

Great post. You mentioned "The cornerstone in the treatment of RSD / CRPS is normal use of the affected part as much as possible." I wholly agree with this. Normal use, touch etc reinfrced the "it's ok" message. Normal movements train the brain just as mirror therapy does. Like Bianca I was two years before diagnosis and ended up with whole body problems. I use mirror therapy with success (I don't use a box. David Butler, who wrote "Explain Pain" with Dr Lorrimer Moseley notes on one of his blogs that an ordinary mirror will do.} Mirror therapy is one very effective way of retraining the brain. There are many other effective ways. If you don't mind my putting a link here Matthais Weinberger has a brilliant series of posts about mirror therapy.http://neurotopian.blogspot.com/2007/11/mirror-box-therapy-part-i.htmlThe Neurotopian: Mirror Box Therapy - Part Ijeiseahttp://www.crps-rsd-a-better-life.blogspot.com

I'm impressed with your knowledge and review about CPRS. About five years ago my spouse fell down some steps, resulting in a wrist fracture, which healed poorly requiring several more surgeries. Shortly after the first surgery her hand and arm turned purple and black (I thought she had gangrene)...We are now five years later. CPRS has changed her life, her psyche, and effecte our entire family. When I hear RSD and CPRS I almost go into a state of fugue. In additon to the signs and symptoms you describe there is also a spatial agnosia. It is exacerbated by the smallest touch, and emotional stress. Minimal trauma (and I do mean minimal) creates purpura,blood blisters, and at times an incredible sheen which is glossy to the point of appearing moist. I also believe there is a central nervous system component and it definitely has humoral components, which alters mood. Lidoderm patches, oral neurontin and/or Lyrica are a great help. Very challenging for the patient.

I'm an RN who has had fibromyalgia with depression and insomnia for ten years. I am waiting for an appt with my MD but think it likely that I've developed CRPS. On 12-27, I developed a severe burning pain in the area round my right medial malleolus (inner ankle bone) with mild edema and warmth to touch. I cannot tolerate it touching my other foot or the mattress at night. The more I am up on it, the more it hurts although there is still pain at rest. I have had another symptom for a number of years that no one could explain. When I raise my left arm and my right arm is at my side, my right hand becomes dusky and tingly with a minute or two. I wonder if this could be related. And in the past few months, I've had intermittent intense pain in both upper arms, unlike typical fibro pain. (My fibro has improved remarkably with guaifenesin and tramalol.) I also have Type 2 diabetes (5 years) and flat feet with a history of heel spurs and plantar fasciitis. Any thoughts on this? Thank you.

There is no cure for CRPS at the present time but early diagnosis and treatment is certainly crucial to limit the disability from the disease. Early treatment, ideally within three months of the first symptoms, often results in remission.

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